A rare presentation of massive hepatomegaly secondary to amyloidosis.

The article focuses on primary systemic amyloidosis presenting as massive hepatomegaly, a rare initial and sole manifestation of this disorder characterized by extracellular deposition of misfolded immunoglobulin light chains (AL amyloidosis). It details a case of a 46-year-old woman with significant liver enlargement, elevated alkaline phosphatase, and cardiac involvement, where diagnosis was confirmed by liver biopsy showing amyloid deposits with characteristic Congo red staining. The report emphasizes that while hepatic involvement is common histologically, symptomatic liver disease is uncommon, and diagnosis often requires tissue biopsy from affected organs after less invasive sites are negative. Treatment options include haematopoietic stem cell transplantation (HSCT) for eligible patients and bortezomib-based regimens for others, highlighting the importance of early recognition and diagnosis to improve outcomes.